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    Pediatric DepartmentResearch Day

    May 9, 2012

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    May 9, 2012

    Welcome to the fourth annual Pediatric Trainee Research Day. It is a pleasure to share with you

    today the work our residents and trainees have been carrying out in childrens health research and

    scholarship.

    As we continue on our journey to creating a world class childrens hospital program, we are

    committed to providing hope to sick children and their families by carrying out the research that will

    improve existing treatments and make the discoveries that will lead to new approaches to pediatric

    diseases.

    To prepare the next generation of pediatricians to take part in this mission, each of our trainees is

    required to carry out a mentored project during their training. Coming from diverse backgrounds they can

    select from a spectrum of projects to best suit their career goals and meet their educational needs. Today

    they will have the opportunity to present their work to their faculty, peers and other colleagues.

    Thank you for joining us and showing support to these young investigators. Special thanks also

    to Dr. Marian Evinger and Dr. Janet Fischel for coordinating the day, Dr. Shane McAllister who helped

    the presenters with their posters, members of the Pediatric Scholarship Oversight Committee, and all of

    the faculty who have served as mentors and provided guidance and encouragement to our trainees.

    Sincerely,

    Margaret M. McGovern, MD, PhD

    Professor and Chair of Pediatrics

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    AGENDA

    8:00 8:20 Sign-in: Wang Center LobbyBreakfast and poster set up: Theater Lobby

    8:20 8:30 Opening remarks: Dean Kaushansky Main Theater

    8:35 9:30 Keynote Address: Martin Pollak, MD, Beth Israel Deaconess Medical Center, Boston

    Why Do African Americans Get So Much Kidney Disease?

    9:30 10:30 Residents Platform Presentations

    Joyce Hui-Yuen, MD STAT3 Mediates a Novel Mechanism to Bypass DNA

    Replication Checkpoint Signaling in Oncogene-Driven Transformation

    Sushma Krishna, MDSingle-course Antenatal Betamethasone Alters Lung

    Morphometry in Late Preterm Lambs

    Shweta Shah, MDMulticystic Dysplastic Kidney: Serial Ultrasound versus VCUG for

    Diagnosing Contralateral VUR

    10:30 10:45 Coffee Break Theater Lobby

    10:45 11:15 Fellows Platform Presentations Main Theater

    Surabhi Jain, MDIntra-nasal Lipopolysaccharide (LPS) Alters Lung Cellular

    Differentiation (CD)14 and Toll-Like Receptor (TLR) 2 Levels in Three-Week Old Rats

    Exposed to Birth Hyperoxia without Affecting CD4 and CD8 positive T cells or TLR 4

    levels

    Shane McAllister, MD, PhDA Restricted Pattern of Serum IgA Reactivity to EBV

    Early Antigen-Diffuse in Hodgkin Lymphoma

    11:30 12:30 Poster Session Theater Lobby

    12:45 1:30 Lunch and Closing Zodiac Gallery (Lower Level)

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    Abstracts

    1. Joyce Hui Yuen

    Resident Platform Presentations

    STAT3 mediates a novel mechanism to bypass DNA replication checkpoint signaling in

    oncogene-driven transformation

    2. Sushma KrishnaSingle-Course Antenatal Betamethasone Alters Lung Morphometry in Late Preterm Lambs.

    3. Shweta ShahMulticystic Dysplastic Kidney: Serial ultrasound versus VCUG for diagnosing contralateral

    VUR.

    4. Surabhi Jain, Neonatology

    Fellow Platform Presentations

    Intra-nasal Lipopolysaccharide (LPS) Alters Lung Cellular Differentiation (CD)14 and Toll-Like

    Receptor (TLR) 2 Levels in Three-Week Old Rats Exposed to Birth Hyperoxia without Affecting

    CD4 and CD8 positive T cells or TLR 4 levels

    5. Shane McAllister, Infectious DiseasesA restricted pattern of serum IgA reactivity to EBV Early Antigen-Diffuse in Hodgkin Lymphoma

    6. RebecccaAbell, Gastroenterology

    Fellow/Faculty Posters

    Magnets Attract Trouble in Teen

    7. SeharEjaz, EndocrinologyCongenital hypothyroidism and anal atresia in a baby with chromosome 10p13 deletion : a case

    report

    8. Janice John, PediatricsDo positive parenting strategies decrease externalizing behaviors? Evidence from the panel study

    of income dynamics.

    9. Julie Khlevner, Gastroenterology

    Establishing Early Enteral Nutrition with the Use of Self Advancing Postpyloric Feeding Tube in

    Critically ill Children

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    10. Sameer Lapsia, GastroenterologyRectal Lipoma in a Pediatric Patient with Inflammatory Bowel Disease

    11. Shane McAllister, Infectious Diseases

    Delayed host cell death during lytic replication of Epstein-Barr virus

    12. NiyatiSkaria, EndocrinologyAccuracy of Continuous Glucose Monitoring in Pediatric Patients with Diabetic Ketoacidosis

    13. Kim Tafuri, EndocrinologyEffect of Pioglitazone on the course of new onset type 1 diabetes

    14. Yury Yakubchyk, Infectious DiseasesExposure to Nanoparticles Increases the rate of Leishmaniasis and Staphylococcus aureus

    Infection

    15.HeelaAziziResident/Student Posters

    Keeping Families Healthy Program: A Look at the Demographic Profile

    16. ErjolaBalliuPrevalence of undiagnosed diabetes among hospitalized patients with hperglycemia

    17. Sandy CheungNormal Anion Gap Metabolic Acidosis: A Case Report

    18. Steven DellAmoreThe Keeping Families Healthy Program: Parent Identification and Management of Overweight

    and Obese Children

    19. MehvishHadiEpstein-Barr Virus-Associated HemophagocyticLymphohistiocytosis (HLH) presenting with

    Renal findings: Case Report

    20. Stephanie HomAsthma, Obesity and Quality of Life

    21. Zak Jacobs

    A Case Report of Severe Mitral Regurgitation Associated with Refeeding Syndrome in an

    Adolescent with Severe Anorexia Nervosa

    22. Parampreet KathuriaNasopharyngeal Teratoma and Imperforate Anus in a Neonate: Case Report

    23. Sushma KrishnaPain-Reducing Interventions During Pediatric Venipuncture and IV Insertion Leads to Increased

    Parent Satisfaction

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    24. Loren MurphySystematic survey of intubation success and opportunity in a duty-hour limited pediatric and

    neonatal training program

    25. Lee PolikoffCharacteristics and outcomes of pediatric rapid response teams before and after automation by a

    pediatric early warning score (PEWS) system

    26. Sheena SharmaSuccessful Remission of Dense Deposit Disease (DDD) with Plasmapheresis: A Case Report

    27. Mike SternThe Stony Brook Parent Handbook: From Birth to 5 Years

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    ABSTRACT 1

    STAT3 mediates a novel mechanism to bypass DNA replication checkpoint signaling in oncogene-

    driven transformation

    Hui-Yuen JS, Koganti S, de la Paz A, Freeman A, Bhaduri-McIntosh S.

    Mechanisms that overcome the intra-S phase DNA replication checkpoint during oncogene-drivenproliferation, allowing successful tumorigenesis, are not well understood. We have previously shown that

    ex vivo infection of primary human B cells with EBV was associated with activation and increasedexpression of signal transducer and activator of transcription-3 (STAT3), a pro-proliferative protein.STAT3 is constitutively active in the majority of human tumors, including EBV-related B cell

    lymphomas. We used Epstein-Barr virus (EBV), a known carcinogen, as a tool to identify mechanismsthat facilitate such bypass of DNA replication checkpoint signaling.

    Background

    Peripheral blood was drawn from EBV-seronegative subjects after obtaining informed consent. Peripheralmononuclear cells were isolated, CD3-positive T cells were depleted, and cells enriched for B cells werecultured for four days in media alone, with EBV, or with EBV plus a chemical inhibitor of STAT3

    phosphorylation. Cells were then harvested and prepared for Western blot and flow cytometry.

    Methods

    We confirmed that ex vivoinfection of primary human B cells with EBV was associated with activationand increased expression of STAT3. When phosphorylation of STAT3 was chemically inhibited duringEBV infection or when B cells from patients bearing naturally-occurring mutations in STAT3gene were

    infected with EBV, proliferation of cells expressing the oncogene Latent Membrane Protein-1 was haltedin S phase of the cell cycle. Moreover, there was absent or delayed emergence of growth-transformed celllines. Compared to un-infected cells, by day four after exposure to EBV, primary B cells demonstrated an

    2600-fold increase in levels of phospho (activated)-ATR, the primary regulator of the DNA replicationcheckpoint pathway. When activation of STAT3 was chemically inhibited, a similar increase in pATR

    was observed along with phosphorylation of Chk1 (pChk1), its downstream target. In contrast, there wasminimal to no pChk1 when B cells were infected in the presence of functional STAT3. Total Chk1 wasrelatively constant regardless of STAT3 function.

    Results

    EBV upregulates STAT3 to suppress levels of pChk1 in response to activated ATR, providing amechanism by which DNA replication checkpoint signaling may be bypassed during oncogene-driven

    transformation.

    Conclusions

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    ABSTRACT 2

    Single-Course Antenatal Betamethasone Alters Lung Morphometry in Late Preterm Lambs.

    Sushma Krishna MD1, Shetal I. Shah MD

    1, Sylvia Gugino MA

    2, SatyanLakshminrusimha MD

    1) Department of Pediatrics, Division of Neonatology, Stony Brook University School of Medicine2

    2) Department of Pediatrics, Division of Neonatology, State University of New York at Buffalo

    Late preterm infants (34 0/7-36 6/7 weeks) exhibit significant rates of respiratory distress syndrome, but

    the use of antenatal steroids at these gestations has not been fully evaluated. The purpose of this study is

    to determine the effect of antenatal betamethasone on lung histology in the late preterm fetal lamb.

    Background

    Time-dated pregnant ewes were injected with betamethasone (15mg/day) or placebo on day of life (DOL)

    132 & 133 (term -147 days) (N=12 each). C-section was performed on DOL 134.

    Methods

    Lungs from 6 lambs from each group were harvested prior to first breath. Six remaining lambs were

    ventilated for 6 hours. Harvested lungs were fixed-inflated and morphometric assessments were

    performed blind on 7m cross sections stained with Hematoxylin-eosin.

    A linear 56-point-counting grid was superimposed onto each digitized image. Air space, alveolar tissue,

    parenchyma, air-tissue intercepts, sampled radial alveolar count, number of alveoli-per-linear field, and

    mean alveolar width were counted. Alveolar density and total alveolar surface area were calculated.

    Full-term, day-old newborn lambs were studied for comparison. One-way ANOVA testing with

    Bonferroni correction was used in analysis.

    Both full-term and steroid-treated lambs exhibited lower radial alveolar counts and decreased alveoli-per-

    linear field measurements compared to control (p

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    ABSTRACT 3

    Multicystic Dysplastic Kidney: Serial ultrasound versus VCUG for diagnosing contralateral VUR

    Shweta Shah, MD1, Margaret Parker, MD1, Catherine Messina, PhD

    Department of Pediatrics, Stony Brook University School of Medicine, Stony Brook, NY.

    1

    Background: Multicystic dysplastic kidney (MCDK) is a congenital anomaly of the kidney with an incidence of

    approximately 1 in 4300 live births and one of the most common anomalies detected by prenatal ultrasound. The

    natural history of these kidneys involves compensatory hypertrophy of the normal kidney, an almost complete

    involution of the affected kidney and preservation of renal function by the solitary kidney. Vesico-uretral reflux

    (VUR) has been found in upto 30% (range 5% to 43%) of MCDK cases and Voiding Cystourethrogram (VCUG)

    has been traditionally used to diagnose the reflux. Recent studies have questioned the utility of routinely performing

    a VCUG, a test that is invasive and involves ionizing radiation, for the diagnosis of the contralateral VUR. The

    MCDK registry data states that the vast majority of the reflux is mild to moderate with no difference in rates of UTI

    or renal scarring between those with and without VUR. A renal US obtained at birth,1month and at 2 years, 5 years

    and 10 years, is reasonable to rule out, initially, findings suggestive of VUR and contralateral kidney abnormalities

    and then, subsequently, to document MCDK involution and evaluate the growth of the contralateral kidney. Routine

    VCUG should be deferred unless abnormal ultrasound features are present in the contralateral kidney or ureter.

    Objective: To determine if doing serial ultrasound in patients with unilateral MCDK yielded same or better results

    than VCUG in diagnosing abnormalities of contralateral kidney/ureter.

    Materials and Method: Our study is a retrospective chart review of all patients with MCDK who were seen in

    Pediatric Nephrology clinic at Stony Brook Medical Center from year March 1995 to March 2012. Those patients

    who had hereditary cystic disease, solitary renal cyst or who did not have a documentation of a VCUG result were

    excluded from the review. An approval for Exemption Review was obtained from Stony Brook University Human

    Subjects Committee (IRB) prior to obtaining the data. In addition to results of serial ultrasound and VCUG, data was

    also obtained for estimating the GFR, degree and presence of proteinuria, hypertension, Wilms tumor and need for

    prophylactic nephrectomy. Data was analyzed by using standard statistical tests.

    Results:32 patients were eligible for the analysis. The number of females were slightly higher than males with the

    M:F ratio of 0.6to 1. There were equal number of patients with right and left MCDK. The Glomerular filtration rate

    ranged from 56-150 ml/min. A prenatal diagnosis of MCDK was made in 81% of the cases. 81% had complete

    involution of their dysplastic kidney. All but 1 patient had compensatory hypertrophy of the normal kidney. 12% of

    the patients had other congenital anomalies of the kidney and urinary tract, primarily duplication of the collecting

    system.2 patients had hypertension that was attributed to their underlying obesity. 2 patients had prophylactic

    nephrectomy of the dysplastic kidney. None of them developed Wilms tumor. Only 1 patient progressed to CKD,

    patient had Gr II reflux into the dysplastic kidney and presence of duplicating collecting system on normal side. 19%

    of the patient had hydronephrosis diagnosed on US. 31% had reflux diagnosed by VCUG, with majority being low

    grades reflux. Out the total number of patients who did not have reflux on VCUG, majority did not have US

    evidence of reflux. 1 patient had hydronephrosis but no reflux.19% had reflux diagnosed by VCUG but had no

    evidence of reflux on US. This were low grade reflux and had self-resolved in subsequent VCUG. None of the high

    grade reflux on VCUG was missed by US. The data were analysed by McNemars test which suggests that reflux is

    identified similarly by both tests because the p value is much greater than 0.05.

    Conclusion:

    US is as reliable as VCUG in detecting high grade reflux in the contralateral kidney in patients with

    MCDK. Low grade reflux may be missed by US, but the majority is self-resolving. Routine VCUG can be deferred

    unless abnormal ultrasound features are present in the contralateral kidney or ureter.

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    ABSTRACT 4

    Intra-nasal Lipopolysaccharide (LPS) Alters Lung Cellular Differentiation (CD)14 and Toll-Like

    Receptor (TLR) 2 Levels in Three-Week Old Rats Exposed to Birth Hyperoxia without Affecting

    CD4 and CD8 positive T cells or TLR 4 levels

    SURABHI JAIN, MD1, SHETAL SHAH, MD1, AVINASH CHANDER, PhD1and PAVAN VASA1

    1Department of Pediatrics, Stony Brook University School of Medicine, Stony Brook, NY.

    Hyperoxia induces endothelial cell injury predisposing newborns to lung inflammation, which contributes

    to bronchopulmonary dysplasia. Premature infants exposed to hyperoxia at birth exhibit increased

    mortality because of late-onset respiratory pathogens or systemic bacteremia, possibly through impaired T

    cell recruitment into the lung. TLR 4 is a membrane receptor which plays a role in CD4

    Background

    +and CD8+T Cell

    recruitment in response to LPS and in anti-oxidant defense. Birth hyperoxia reduces lung TLR4 levels and

    CD4+T cells immediately post-exposure. Intra-nasal LPS increases the acute systemic cytokine response

    following birth hyperoxia. However, the lung immune response after late-onset infection following birth

    hyperoxia is not known.

    The purpose of this study was to determine the long-term effect of birth hyperoxia on lung cells

    expressing TLR4, TLR2, CD4, CD8, CD3, CD14 and CD45 proteins.

    Objective

    Newborn rat pups were exposed for 24 hours to 100% O

    Design/Methods

    2(hyperoxia) or room air. Animals were returned

    to normoxia for 3 weeks, when all animals received intra-nasal LPS (10ug) or saline (Control). Lungs

    were harvested 24 hours later and analyzed by immunohistochemistry for indicated proteins. Quantitative

    microscopy was performed from 30-40 images in each case. Results were normalized for cell number.

    Statistical analysis was performed by ANOVA or Student's t test. P

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    ABSTRACT 5

    A restricted pattern of serum IgA reactivity to EBV Early Antigen-Diffuse in Hodgkin Lymphoma

    Shane McAllister1, Marisa Rotenberg

    2, Duane Shedd

    3, Nancy Mueller

    4, Ellen Chang

    5, George Miller

    6,

    and Sumita Bhaduri-McIntosh

    1

    1Division of Pediatric Infectious Diseases, Stony Brook Long Island Childrens Hospital and Stony Brook

    University School of Medicine, Stony Brook, NY; 2Department of Genetics, Yale University School ofMedicine, New Haven, CT;

    3Department of Pediatrics, Yale University School of Medicine, New Haven,

    CT; 4Department of Epidemiology, Harvard School of Public Health, Boston, MA;5Northern California

    Cancer Center, Fremont, CA, and Division of Epidemiology, Department of Health Research and Policy,

    Stanford University School of Medicine, Stanford, CA; 6Departments of Pediatrics, Molecular Biophysicsand Biochemistry, and Epidemiology and Public Health, Yale University School of Medicine, NewHaven, CT; 7

    Division of Pediatric Infectious Diseases, Stony Brook Long Island Childrens Hospital;

    Molecular Genetics and Microbiology, Stony Brook University School of Medicine, Stony Brook, NY

    Hodgkin lymphoma (HL) is associated with elevated serum antibodies to Epstein-Barr virus (EBV) lyticantigens. Evidence for EBV lytic reactivation during HL, likely in the oropharyngeal mucosal

    compartment, led us to hypothesize that sera from HL patients containIgA to early-lytic antigens,essential for viral DNA replication. Furthermore, we asked if IgA to EBV early-lytic antigens can

    differentiate between sera from patients with HL and acute infectious mononucleosis (IM).

    Background

    Convenience serum samples from 42 previously described patients with HL, 17 IM, and 15 healthy EBV-positive controls were obtained via IRB-approved protocols. Lytic replication was induced with sodium

    butyrate +/- phosphonoacetic acid and lytic antigen expression was detected by flow cytometry. Totalserum IgA was detected by ELISA. The specificity of serum IgA was determined by immunoblottingrecombinant EBV proteins.

    Methods

    We found that 95.2% and 71.4% of HL patients, half with EBV-positive tumors, contained IgA directedagainst cells expressing EBV total- and early-lytic antigens, respectively. IgA in healthy EBV-seropositive sera did not recognize total-lytic antigen expressing cells; however, 20% healthy sera weakly

    detected early-lytic antigen expressing cells. The immunoblot pattern of IgA specific for EBV EarlyAntigen-Diffuse reliably differentiated between patients with IM and HL irrespective of EBV in tumors.

    The sensitivity of this pattern for distinguishing HL from IM was 92.9%, specificity 100%, positivepredictive value 100%, and negative predictive value 83.3%.

    Results

    The diagnostic immunoblot pattern described here may distinguish acute IM from HL, two EBV-related

    diseases that can present similarly. The robust performance characteristics of our test should be evaluated

    further to ascertain its utility as a predictive biomarker for development of HL and response to

    chemotherapy.

    Conclusions

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    ABSTRACT 6

    Magnets Attract Trouble in Teen

    RebecccaAbell, DO, Thomas Lee, MD, Richard Scriven, MD, George Angelos, MD, and Jeffrey

    Morganstern, MD

    Foreign body ingestion is extremely common in the pediatric population. Of all foreign bodies ingested,

    80% will pass spontaneously, 10-20% will require endoscopic removal, and 1% will need surgical

    intervention. With the increasing number of magnetic pieces in toys and household items, the number of

    magnetic ingestions is escalating, although still rare overall. These become difficult cases to manage

    because the history is often unclear. Magnetic ingestion can cause significant morbidity and mortality if

    not managed in a timely manner. Any magnetic ingestion involving more than one magnet or a magnet

    and a metallic objectshould prompt immediate management.

    Introduction

    A 14 year old female presented with acute abdominal pain. Imaging of her abdomen demonstrated 2

    circular foreign bodies in the right upper quadrant and 3 similar foreign bodies in the right lower

    quadrant. The patient reported accidentally swallowing magnets after trying to create the illusion of a

    tongue ring. After 5 days without progression, pediatric gastroenterology was consulted.

    Esophagogastroduodenoscopy and colonoscopy were performed. No magnets were visualized, but upon

    removal of the upper endoscope, 2 magnets were found on the shaft of the instrument. Post-procedure, the

    patient presented with signs of perforation. She was taken to surgery, where jejunal perforation and 3

    magnets embedded in the cecal wall were found. She had resection and anastamosis of both areas. The

    patient did well post-operatively.

    Case

    To our knowledge, this is the first reported case of a magnetic object being attracted to the endoscope

    prompting its removal. It is well known that the ingestion of multiple magnets may be associated with

    significant morbidity due to perforation. Early intervention by endoscopy or surgery may be necessary,

    depending on the number of magnets ingested, time since ingestion, and anatomical location. If

    endoscopy is undertaken, the gastroenterologist should be aware of the ferromagnetic properties of the

    endoscope.

    Conclusions

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    ABSTRACT 7

    Congenital hypothyroidism and anal atresia in a baby with chromosome 10p13 deletion : a case

    report

    SeharEjaz, MD1

    , Kimberly Tafuri, MD1

    , Andrew Lane, MD1

    , Patricia Galvin-Parton, MD1

    and Thomas AWilson, MD1.

    1Department of Pediatrics, Stony Brook University School of Medicine, Stony Brook, NY, United States.

    22q11DS (DiGeorge syndrome, MIM 188400) is a developmental defect of the 3rd and 4th pharyngeal

    pouches associated with facial dysmorphism, conotruncal heart defects and absent /hypoplastic thymus

    and parathyroid. Deletion of chromosome 10p14-13 presents with similar defects, hence characterized as

    DiGeorge syndrome 2 (DGS2-MIM 601362). Congenital hypothyroidism has been reported with 22q11

    deletion secondary to T-box transcription factor TBX1deletion (MIM# 602054). To our knowledge, thisis the first case of congenital hypothyroidism reported in DGS2.

    Background

    A full term Caucasian baby boy was born with anal atresia after an uneventful pregnancy to a 33 yrs old

    mother. Birth weight was 2.3 kg and length 48 cm. The initial newborn screening suggested

    hypothyroidism and decreased T cell subsets. Hypothyroidism was confirmed (TSH 635 mcu/ml, total T4

    2.1 mcg/dl). Hypocalcemia (Ca 7.7 mg/dl, iCa 3.9 mg/dl) developed on day 2 of life. Baseline EKG

    showed right ventricular hypertrophy with non specific depolarization anomaly. No structural cardiac

    anomalies were detected on echocardiogram. Karyotype was 46, XY, del(10)(p13). Levo-thyroxine andcalcium carbonate corrected the hypothyroidism and hypocalcemia. Sensorineural hearing loss was

    confirmed with BAER after he failed an initial hearing screen.

    Case

    DGS2 is usually associated with hypoparathyrodism, deafness and renal dysplasia (HDR/Barakat

    syndrome) due to associated GATA3 mutations/deletions. Although congenital hypothyroidism and anal

    atresia in our patient might be coincidental, it may also indicate the role of yet unknown genetic

    mechanisms responsible for the association of CH with DiGeorge syndrome 2. TBX1 gene deletion is

    thought to be responsible for thyroid dysgenesis with 22q11 deletion; however, whether TBX1 plays a

    role in the hypothyroidism of this patient with DGS2 is unclear.

    Interestingly, the mother carried a diagnosis of Brugada syndrome and has an internal pacemaker. She has

    not had genetic testing to evaluate the mutations associated with Brugada syndrome. We speculate that

    the mother might have Brugada subtype 4 secondary to CACNB2 deletion located on 10p12.33-10p12.31

    due to close proximity to GATA3 and DGS2 gene loci; however, the genetic results from parents are not

    available at this point.

    Discussion

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    ABSTRACT 8

    DO POSITIVE PARENTING STRATEGIES DECREASE EXTERNALIZING BEHAVIORS?

    EVIDENCE FROM THE PANEL STUDY OF INCOME DYNAMICS1Janice Thomas John DO, MS, MPH,

    1Susmita Pati, MD, MPH, and

    2Tia M. Palermo, PhD

    1Department of Pediatrics and 2Department of Preventive Medicine, Stony Brook University School ofMedicine

    Externalizing behavioral problems in childhood have potentially severe, long term consequences.

    Over the last decade, there has been a significant rise in the prevalence of these behaviors.

    Previous studies evaluating the relationship between parenting strategies and externalizing

    behaviors are limited in that they are dated, limited in geographic scope and do not provide

    longitudinal data. This study examines the relationship between parenting strategies and

    externalizing behaviors in children.

    Introduction

    We analyze data on 1,176 children in Waves II and II (2002 and 2007) of the Child Development

    Supplement of the Panel Survey of Income Dynamics. Externalizing behavior problems were

    operationalized using the Behavior Problem Index. Independent variables included parental

    warmth,television exposure, discussing household rules and limits, disciplining styles and other

    covariates. We performed pooled and longitudinal analyses using multivariate linear

    regressions.

    Methods

    Children who experienced more parental warmth had lower externalizing behavior problemsubscale scores in the subsequent wave (p

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    time, increasing parental warmth, teaching healthy disciplining styles and other beneficial

    parenting strategies.

    ABSTRACT 9

    Establishing Early Enteral Nutrition with the Use of Self Advancing Postpyloric Feeding Tube inCritically ill Children

    Julie Khlevner, MD1, Janice Antino,RD,MS,CSP2, Rahul Panesar, MD3, and Anupama Chawla, MD1

    1Division of Pediatric Gastroenterology, Hepatology and Nutrition, Stony Brook University School of

    Medicine;2Division of Dietary Services, Stony Brook University School of Medicine;3Division of

    Pediatric Critical Care Medicine, Stony Brook University School of Medicine

    Early nutrition support is an integral part of the care of critically ill children. Early enteral nutrition (EN)

    improves nitrogen balance and prevents bacterial translocation and gut mucosal atrophy. Adequate EN isoften not achieved as gastric feeds are not tolerated and placing postpyloric feeding tubes can be difficult.

    Spontaneous transpyloric passage of standard feeding tubes without endoscopic intervention or use of

    anesthesia can range from 30% to 80%. We report on our experience with a 14Fr polyurethane self-

    advancing jejunal feeding tube in a pediatric population. These tubes have been used in the adult

    population with successbut to our knowledge there have been no reports of its use in the pediatric age

    group.

    Introduction

    We present 7 critically ill patients 8-19 years old, admitted to pediatric intensive care unit, in whom

    prolonged recovery, inability to tolerate gastric feeds and dependence on ventilator was predicted at theoutset. The jejunal feeding tube was successfully placed on first attempt at the bedside in all of the 7

    patients within the first 24hrs without the use of a promotility agent or endoscopic intervention.

    Nutritional goal achieved within 48hrs of feeding tube placement was reported for each patient. Our case

    series demonstrates that children fed via the small bowel reached their nutritional goal earlier and did not

    require parenteral nutrition.

    Case Series

    The self-advancing jejunal feeding tube can be used effectively to establish early EN in critically ill

    children.

    Conclusions

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    ABSTRACT 10

    Rectal Lipoma in a Pediatric Patient with Inflammatory Bowel Disease

    Sameer Lapsia, MD, Julie Khlevner, MD, Jeffrey Morganstern, MD, and Anupama Chawla, MD

    Pediatric Gastroenterology, Stony Brook University School of Medicine, Stony Brook, NY.

    Gastrointestinal manifestations of inflammatory bowel disease, especially Crohns disease, includeinflammation, edema, pseudopolyps, strictures, and stenosis.

    Background

    We present a case of a rectal lipoma in a patient with inflammatory bowel disease and one of very fewcases reported in children.

    Objective

    Patient is a 16 year old female with Crohns disease complicated with perineal fistulas, joint pain,episcleritis, and erythema nodosum. Due to worsening disease despite being on maximized medicaltherapy such as Humira, a TNF antagonist, the patient underwent a colonoscopy to assess the full extent

    of her disease and determine if surgical intervention would be an option. At approximately 10 cm fromthe anal opening, a smooth, polypoid mass 1.5 cm in diameter with a 1 cm base was noted protruding into

    the lumen. Due to concerns for a possible cancerous lesion, the polypoid mass was resected. Pathologydiagnosed the mass to be a lipoma. No malignant characteristics were noted.

    Design/Method

    Colonic lipomas are soft tissue tumors originating from mature adipocytes. Although rare, they are thesecond most common benign tumor of the colon after adenomatous polyps with an incidence ranging

    between 0.2% and 4.4% with no cases reported in patients with inflammatory bowel disease. Resection of

    these masses is essential due to complications arising from lesions larger than 2 cm such as abdominalpain, change in bowel pattern, massive bleeding, obstruction, intussusception, or perforation. Cases ofthese lipomas metamorphosing into liposarcomas have been reported making removal of this lesion anecessity. The large base of the mass in our patient warranted a 2-step procedure, firstly achievinghemostasis with the endoloop followed by snare cauterization of the polypoid mass.

    Results

    Lipomas must be considered as part of the differential diagnosis when a colonic polypoid lesion isencountered in patients with inflammatory bowel disease.

    Conclusions

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    ABSTRACT 11

    Delayed host cell death during lytic replication of Epstein-Barr virus

    Shane McAllister1and Sumita Bhaduri-McIntosh1, 2

    1Department of Pediatrics, Stony Brook Long Island Childrens Hospital and 2

    Department of Molecular

    Genetics and Microbiology, Stony Brook University, Stony Brook, NY.

    Periodic reactivation of the tumorigenic herpesvirus Epstein-Barr virus (EBV) from latency results in the

    release of progeny virus. This lytic reactivation is important in the pathogenesis of EBV-mediated

    diseases such as lymphomas and lymphoproliferative diseases that arise during immunocompromise.

    Detection of lytic viral DNA and proteins is an important component of host innate and adaptive defense

    mechanisms. While on the one hand, attempts are made to stall production of infectious virus and pro-

    apoptotic signals are delivered within the infected cell, EBV is able to stave off apoptotic death of the

    host cell by expressing anti-apoptotic viral genes and inducing expression of anti-apoptotic and pro-

    survival cellular genes. Delay of host cell death allows for replication and release of progeny virus. The

    mechanisms contributing towards such delayed cell death in lytically infected cells has received little

    attention.

    Background

    LCLs were established by infecting peripheral blood mononuclear cells with EBV in the presence of

    Tacrolimus to prevent T cell-mediated killing of EBV-infected B cells. DNA content of LCL was

    determined by propidium iodide staining followed by flow cytometry. DNA content of lytically-infected

    LCL was correlated with early or late lytic cycle by co-staining for Early Antigen-Diffuse (early lytic

    cycle) or gp350 (late lytic cycle). Autophagy was identified in LCL by immunofluorescent detection of

    LC3. Nuclear morphology of lytically-infected LCL was determined by immunofluorescent staining foreither EA-D or gp350 with counterstaining of host-cell DNA with DAPI.

    Methods

    LCL expressing surface gp350 had condensed nuclei consistent with cells undergoing the nuclear

    reactions of apoptosis. Notably, many of these intact cells appeared to have fractional nuclei when

    examined by immunofluorescence. Flow cytometry-based examination of DNA content confirmed that

    while all cells expressing EA-D contained 2N to 4N DNA, approximately 25% of live, gp350-positive

    late lytic cells contained less than 2N DNA. Simultaneous staining with Annexin V revealed a progressive

    increase in the fraction of Annexin V-positive lytic cells in early, intermediate lytic, and late lytic stage

    cells. Autophagic vesicles were identified in lytically-infected LCL.

    Results

    Together, these results indicate that signs of cell stress are demonstrable upon lytic reactivation and

    worsen as lytic replication proceeds. Further studies in our laboratory are aimed at characterizing the cell

    death pathways activated by lytic replication of EBV and delineating viral mechanisms that are necessary

    to delay host cell death.

    Conclusions

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    ABSTRACT 12

    Accuracy of Continuous Glucose Monitoring in Pediatric Patients with Diabetic Ketoacidosis

    NiyatiSkaria MD, SeharEjaz MBBS, Kimberly Tafuri DO, Thomas Wilson MD, and Andrew Lane MD

    Continuous glucose monitors (CGM) are increasingly used in the outpatient setting. Patients may make

    clinical decisions based on CGM readings, including during development of diabetic ketoacidosis (DKA).

    Because CGM devices rely on measurement of generated hydrogen ions, it is unknown whether DKA

    affects CGM accuracy.

    Background

    We aim to determine whether a commercially available CGM (Dexcom) measures blood glucose

    measurements accurately compared to a standard of care bedside glucometer in the setting of DKA.

    Objective

    Succesive eligible patients ages 2 to 21 years old admitted to our hospital in DKA (venous pH .05). In a

    Clark Error Grid Anlaysis- 45% of values fell in category A- desirable correlation and 44% in category B-

    acceptable correlation. A total of 89% fell in category A and B. Of the rest of the values, 9% fell in

    category C- risk for inappropriate treatment, 2% in categories D and E- high risk.

    Results

    Other investigators studying correlations between CGM and reference values in healthy individuals with

    diabetes have demonstrated MARDs of 13.2 to 21.5%, and 98% of values falling within Clarke Error

    Grid category A and B. In contrast, our data suggest that during DKA, there is a much higher MARD

    between CGM and reference values, and more values fall outside Clarke categories A and B. Our resultshave important implications in counseling patients and caretakers regarding reliance on CGM values at

    times of risk for the development of diabetic ketoacidosis.

    Conclusion

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    ABSTRACT 13

    Effect of Pioglitazone on the course of new onset type 1 diabetes

    Kimberly Tafuri, DO, MustaqGodil, MD, Andrew Lane, MD and Thomas Wilson, MD.

    Type 1 diabetes (T1DM) is caused by insulin deficiency resulting from progressive destruction of insulin

    producing beta cells. The histological hallmark of the diabetic islet is mononuclear cell infiltration or

    insulitis. Thiazolidinediones (TZDs) activate PPAR & enhance the actions of insulin. Studies in the non

    obese diabetic &streptocotozin treated mouse models demonstrated that pretreatment with TZDs

    prevented or delayed the development of T1DM.

    Background

    This study examines whether the TZD, pioglitazone (PIO), given with insulin, preserves beta cell function

    in patients with new onset T1DM.

    Objective

    This is a prospective, randomized, double-blind, placebo controlled 24 week study in patients with new

    onset T1DM. Subjects received either PIO or placebo. A total of 15 patients were enrolled. Eligibility

    criteria included: T1DM, age > 6 years, & absence of liver disease. Blood sugar, HBA1C, C-peptide, &

    liver enzymes were measured at baseline. Boost stimulated C-peptide responses were measured at

    baseline & 24 weeks. Patients were followed at 2 and 4 weeks and monthly therafter. Blood sugar, insulin

    dose, height, weight & hepatic enzymes were monitored at each visit. HBA1C was performed every 12

    weeks.

    Design/Methods

    Of the 15 patients, 8 received PIO and 7 placebo. 6/8 patients in the PIO group completed the study. 1

    was removed from the study because of elevated transaminases (transient) and 1 withdrew. Age at entry

    & duration of diabetes (approximately 3 months) was similar between groups. Baseline daily insulin dose

    was higher in the PIO group (0.46 vs. 0.28 u/kg/d); HBA1C was slightly lower in the PIO group (6.7%

    vs. 7.9%). Mean peak C-peptide values were similar between groups at baseline (PIO: 1.6 vs. Placebo:

    1.54 ng/ml). Mean peak C-peptide was slightly greater at 24 weeks in the PIO group compared to placebo

    (1.75 vs 1.5 ng/ml) which is thought to be clinically insignificant. The interaction of HBA1C and insulin

    dose (HBA1C* insulin/kg/day), which combines degree of diabetic control and dose of insulin required toachieve this control, showed transient improvement in the PIO group at 12 weeks but was not sustained at

    24 weeks, whereas the placebo group demonstrated a progressive increase throughout the 24 weeks.

    Results

    In this pilot study, PIO did not appear to preserve beta cell function when compared to placebo. Our

    negative results may be impacted by the small sample size.

    Conclusions

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    ABSTRACT 14

    Exposure to Nanoparticles Increases the rate of Leishmaniasis andStaphylococcus aureusInfection

    Yury Yakubchyk, Miriam H. Rafailovich

    Leishmaniasis is a vector borne parasitic disease affecting millions of adults and children, mainly in

    underdeveloped countries. Cutaneous form of this disease is presented as chronic skin ulcerations with

    primary involvement of macrophages, where parasite reproduces and propagates. Often these cutaneous

    lesions are infected with pathogenic microflora, including Staphylococcus aureus(S. aureus) as a

    common commensal of skin and mucous membranes. TiO

    Background

    2and ZnO nanoparticles (NPs) due to their

    known anti-bacterial action are often applied to the skin in different formulations (sunscreens, ointments

    and creams). These NPs can also penetrate the cell membrane and alter multiple cellular functions. We

    studied the effect and influence of TiO2and ZnO NPs onLeishmaniatropica(L. tropica) parasites and on

    the response of J774A.1 murine macrophages to infection byL. tropicaand S. aureus.

    L. tropicaparasites and murine J774A.1 macrophages separately were exposed to 0.01 mg/ml of

    TiO

    Methods

    2and ZnO NPs for 1 hour with subsequent assessment of parasites and cells proliferation via light

    microscopy. J774A.1 cells were exposed to 0.01 mg/ml NPs for 1 hour or 24 hours and infected withL.

    tropicaor opsonized S. aureusrespectively. Infected murine macrophages cultures were incubated for 24

    hours with following fixation and staining with Giemsa. The percentage of infected cells and the intensity

    of infection (number of parasites per 100 total host cells) were determined by light microscopy.

    Transmission Electron Microscopy (TEM) was performed to reveal the localization of TiO2and ZnO NPs

    in exposed to NPs and infected J774A.1 cells.

    We found no effect on parasite and cell proliferation after 1-hour exposure to 0.01 mg/ml TiO

    Results

    2or ZnO

    NPs. Intensity of infection of NP-exposed macrophages withL. tropicawas 2 to 6 times greater than in

    the control group (p

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    ABSTRACT 15

    Keeping Families Healthy Program: A Look at the Demographic Profile

    HeelaAzizi, Janice John, Kristi Ladowksi, andSusmitaPati

    Department of Pediatrics, Stony Brook University School of Medicine

    The programs study aims to identify the effectiveness of home visits in improving patient compliance

    and childhood immunization in a pediatric population from birth through 17 years of age. Created by

    pediatricians at Stony Brook Childrens Hospital, Keeping Families Healthyis a free home visitation

    program designed to help families receive information and resources needed to make the best decisions

    regarding their childs health. The program acts as an extension of the pediatricians office by

    communicating families health questions with their childs doctor and navigating the healthcare system.

    The program has 3 main areas of focus; management of chronic diseases, well child/adolescent care, and

    newborn care. In an observational study, a target of 1300 individuals from the SBUH Tech Park Clinic is

    being recruited as the control group. Another 1300 individuals from the Patchogue, NY area are expected

    to be enrolled in the home visitation program as the intervention group. The trained community health

    workers tailor each familys visit to their childs specific needs using 7 program protocols: Newborn,

    Premature Gestation, Asthma, Diabetes, Obesity, Other Chronic Conditions, and Healthy Adolescents.

    While this study is ongoing, the 2 aims of my research will be (1) to determine how well the Tech Park

    enrollees function as a control for the intervention group, and (2) to identify the types of families likely to

    participate in a home visitation program, based on their demographics. These results will help us better

    understand what populations to target for similar home intervention programs in the future.

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    ABSTRACT 16

    Prevalence of undiagnosed diabetes among hospitalized patients with hperglycemia

    1,2Erjola Balliu, MD,

    2Marina Charitou, MD,

    2

    Shai Gavi DO

    1

    Department of Pediatrics and2

    Department of Internal Medicine, Stony Brook University School ofMedicine, Stony Brook, NY.

    Early detection of diabetes can significantly alter the disease progression. Our objective was to screenhyperglycemic patients and identify those with undiagnosed diabetes using a hemoglobin A1c test. Inaddition, other causes of hyperglycemia were further characterized such as pre-diabetes, stresshyperglycemia and steroid induced hyperglycemia.

    Introduction

    We randomly selected 611 patients admitted to the General Medicine Floors, the Coronary Care Units

    (CCU), and the Intensive Care Units (ICU) at Stony Brook University Hospital. Patients with randomblood glucose over 200mg/dL were identified and a hemoglobin A1c test was performed on each patient.A diagnosis of undiagnosed diabetes was made if the hemoglobin A1c was greater than or equal to 6.5%and there was no prior history of diabetes. Patients with HbA1c 5.7-6.4% were identified as pre-diabetes.In addition, the prevalence of stress hyperglycemia and steroid induced diabetes was also made.

    Methods

    148/611 (24%) patients had elevated random blood glucose levels > 200 mg/dL. Upon review of these

    patients' medical records, 9/148 (6.1%) were found to have an elevated HbA1c 6.5% without a previousdiagnosis of diabetes, while 10/148(6.8%) were found to have HbA1c 5.7- 6.4% and were classified as

    pre-diabetes. 22/148 (15%) were found to have elevated serum glucose levels with HbA1c < 5.7%. Of the

    latter group, 17 patients (11.5%) were on steroid therapy and 5 patients (3.4%) were categorized as stresshyperglycemia. There was an increased prevalence of undiagnosed diabetes in the ICU/CCU in

    comparison to the General Medicine Floors (18.8% vs. 4.9%, respectively).

    Results

    Recent reports have documented a prevalence of undiagnosed diabetes in the inpatient population as high

    as 18%, our study shows a prevalence of 6.1%among hyperglycemic patients, with an increasedprevalence in patients admitted to the CCU/ICU compared to the General Medicine Floors.

    Conclusion

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    ABSTRACT 17

    Normal Anion Gap Metabolic Acidosis: A Case Report

    Sandy Cheung1, DO, MPH, Rachel Boykan1, MD1Department of Pediatrics, Stony Brook University School of Medicine, Stony Brook, NY

    Milk and soy are the most common food triggers of food protein enterocolitis syndrome (FPEC) in young

    infants, and up to 50% of those with milk protein allergy also react to soy. The incidence of FPEC

    attributed to milk protein is reported between 0.35-2%. In the case of milk and soy-induced FPEC, the

    majority of reactions are thought to be non-IgE-medicated.

    Background

    We present the case of a 6 week old African American male with diarrhea and dehydration.

    Objective

    Our patient presented with one-day history of projectile, non-bilious, non-bloody vomiting, non-bloody

    diarrhea and decreased oral intake with weight loss of 570 grams over the past week. The infant was

    recently switching from regular formula to lactose free formula. Laboratory studies on admission reveal a

    metabolic acidosis. After 24 hours of rehydration, switched to a casein hydrolysate formula, emesis has

    resolved, the diarrhea has lessened, however the normal anion gap metabolic persists. Evaluation for

    amino and organic acidemias was negative. Given the persistent of a normal anion gap metabolic

    acidosis in the setting of improving diarrhea, the working diagnosis is a renal tubular acidosis.

    Methods

    Renal studies including ultrasound reveal both kidneys to be within the normal limits. Subsequent follow

    up reveals the true diagnosis of food protein enterocolitis syndrome. The patient was then switched to an

    amino acid based formula. At subsequent follow up, his diarrhea had completely resolved, as had the

    anion gap acidosis and hypokalemia.

    Results

    In most of milk protein allergy, the diagnosis is made by elimination of exposure to milk protein, by

    limiting ingestion in breast feeding mothers, or changing to a hydrolyzed casein formula. Stool occult

    blood is often positive, even in the absence of grossly bloody stools. In severe cases, a more extensivelaboratory work up may be warranted, especially if other conditions must be excluded. In the case of

    milk and soy-induced FPEC, the majority of reactions are thought to be non-IgE mediated. 50-80% of

    children diagnosed with FPEC tolerated milk-based foods after a year of life.

    Conclusions

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    ABSTRACT 18

    TheKeeping Families HealthyProgram: Parent Identification and Management of Overweight and

    Obese Children

    Steven DellAmore; Kristi Ladowski, MPH; Janice John, DO, MS, MPH; SusmitaPati, MD, MPH

    Stony Brook University School of Medicine

    Childhood overweight and obesity are increasing in prevalence. The early diagnosis of childhood obesity

    is important to prevent future health problems. Keeping Families Healthy(KFH) is a free service

    provided by the Stony Brook Long Island Childrens Hospital. KFH sends trained community health

    workers (CHWs) into the home for scheduled visits. Keeping Families Healthy acts as an extension of the

    pediatricians office by offering enriched health education. Among other topics, CHWs focus on

    nutrition, activity, and weight management. KFH CHWs are trained to take the heights and weights of

    participants and calculate BMIs and percentiles. Once a child is identified as overweight or obese, based

    on their BMI percentile, the CHW asks specific questions about the childs lifestyle. We hypothesize that

    most parents do not identify overweight/obesity as a medical condition. We also hypothesize that the

    children of these parents will be less active and have less healthy diets when compared with the children

    whose parents identified overweight/obesity as being a medical condition. This study includes 62 children

    enrolled in the KFH program, ages 2 to 17 years, who have a BMI at or above the 85 th

    percentile. Upon

    initially signing up for the program, parents were asked if their child has any pre-existing medical

    conditions. Of the 62 overweight/obese children, 21% of their parents identified their child as having an

    overweight/obesity medical condition. This finding supports our hypothesis that many parents have an

    inaccurate perception of their childrens health. Furthermore, this finding demonstrates the need for early

    identification and education for both parents and their children so that their overweight state or obesity

    can be addressed by modifying nutrition, eating habits and other aspects of lifestyle. Data analysis is

    currently being conducted and we anticipate that the results will support out hypotheses. The results fromthis study are critical, as they may impact future primary care practices.

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    ABSTRACT 19

    Epstein-Barr Virus-Associated HemophagocyticLymphohistiocytosis (HLH) presenting with Renal

    findings: Case Report

    MehvashHadi DO, Julie Cherian MD

    1Department of Pediatrics, Stony Brook University School of Medicine and

    2Division of Pediatric

    Rheumatology, Department of Pediatrics, Stony Brook University School of Medicine, Stony Brook NY

    Hemophagocyticlymphohistiocytosis (HLH) is a potential life threatening syndrome in which the body is

    in a state of uncontrolled inflammation. The main features of this are impaired function of natural killer

    (NK) cells and cytotoxic T cells (CTL). There is uncontrolled accumulation of activated T-lymphocytes

    and activated histiocytes. An important aspect of HLH is cytokine dysfunction. HLH is either due to

    familial (primary HLH) or associated with infection, autoimmune or malignancies (secondary HLH ).

    Criteria for diagnosis include the following signs and symptoms: fever, cytopenia in two cell lines,splenomegaly, hypertriglyceridemia and or hypofibringinemia, hyperferritinemia, and demonstration of

    hemophagocytosis. An established treatment protocol exist for patients diagnosed with HLH, this

    protocol has significantly reduced mortality in children diagnosed with HLH. The HLH-2004 protocol

    consists of therapy over an 8-week period consisting of dexamethasone, etoposide, and cyclosporine +/-

    intrathecal methotrexate. EBV is the most commonly identified trigger in secondary HLH.

    Background

    We present an unusual case of EBV associated HLH and nephrotic syndrome. To the extent of our

    knowledge this is the first case report of EBV associated HLH presenting with nephrotic syndrome. We

    describe a case of a 16 year old female that presents with 1 month history of persistent fevers and malaisethat was diagnosed with infectious mononucleosis via mono spot test. She subsequently developed a

    diffuse maculopapular rash, jaundice, lower extremity edema, low urine output and with a

    hepatosplenomegaly.

    Case

    It is important to have a high index of suspicion for macrophage activation syndrome (MAS) on

    presentation as some authors consider it secondary HLH. MAS is a life threatening syndrome seen in

    patients with systemic juvenile idiopathic arthritis (systemic JIA) and systemic lupus erythematous (SLE).

    Although no treatment protocol is established for MAS, standard of treatment currently consist of

    corticosteroids +/- cyclosporine. Recent studies have reported positive outcomes with the use of Anakinrawhich is a soluble IL-1 receptor antagonist. Anakinra has fewer side effects and is a highly effective

    treatment.

    Discussion

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    ABSTRACT 20

    Asthma, Obesity and Quality of Life

    S Hom, C Messina, F Qureshi, C Kier

    Quality of life (QOL) measures have recently been included in outcome studies, emphasizing both

    medical scientific and patient-related outcomes. Both asthma and obesity pose significant limitations on

    QOL. In addition, there is literature to support a possible link between obesity and the development of

    asthma. Nonetheless, there is limited data evaluating the QOL of obese asthmatic and non-asthmatic

    subjects with their normal weight counterparts. It is also unclear whether patients who are obese and

    asthmatic have poorer QOL compared to normal weight asthmatics.

    Background

    A preliminary study comparing QOL differences between obese asthmatics and non-asthmatics

    (Kier protocol Asthma Obesity) demonstrated mean QOL scores that were lower in the obese asthmatic

    group; however, these results were not statistically significant. This study increases sample size by

    extending QOL measures from the original pilot study to other subpopulations, including normal weight

    asthmatics and non-asthmatics.

    A goal of 80 subjects between 8-17 years of age was to be recruited from Stony Brook outpatient

    facilities. Children were screened for eligibility by completion of anintake form, which reviewed

    previously-made diagnoses of asthma by a physician, current medications, and co-morbid medical

    conditions, as well as the childs height, weight, and calculated body mass index (BMI). Subjects and

    their parents were then distributed standardized PedsQL 4.0 questionnaires pertaining to general health

    and/or asthma, depending on the subject.

    Methods

    Enrollment of subjects was challenging due to limited encounters with patients that met eligibility criteria,

    in particular the age cut-off. Statistical analysis of patient data using one-way analysis of variance

    (ANOVA) and Dunnett tests is still ongoing at the time of this abstract.

    Results

    Analysis of patient data is ongoing at the time of this abstract. Continuing enrollment will enable us to

    further elucidate differences between obese/normal weight asthmatics from obese/normal weight non-

    asthmatics. Identifying these differences in QOL inventories will allow us to tailor diagnosis and

    management for these subpopulations of patients. Furthermore, it will enable us to target individuals at

    risk for poor compliance to provide them with specialized education and guidance.

    Conclusions

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    ABSTRACT 21

    A Case Report of Severe Mitral Regurgitation Associated with Refeeding Syndrome in an

    Adolescent with Severe Anorexia Nervosa

    Zachary Jacobs, DO1, Allison Eliscu, MD2

    1Department of Pediatrics and

    2Division of Adolescent Medicine, Stony Brook University School of

    Mecidine, Stony Brook, NY

    BackgroundAnorexia Nervosa is a well-known disorder with a multitude of well-known medical

    complications due to starvation and extreme malnourishment. Complications are varied and commonly

    include electrolyte abnormalities, refeeding syndrome, amenorrhea, anemia, and cardiac abnormalities,

    most commonly bradycardia. Refeeding syndrome is encountered during the reintroduction of feeding

    after a period of starvation and is also associated with various medical complications, commonly cardiac

    and neurologic. Although arrhythmias and cardiac failure have been documented as complications from

    refeeding syndrome, there have been no documented cases of significant mitral regurgitation secondary to

    refeeding syndrome.

    ObjectiveWe present a case of a 16 year old Caucasian female with severe anorexia nervosa who

    developed refeeding syndrome early in hospital treatment and subsequently developed moderate-to-severe

    mitral regurgitation.

    MethodsOur patient, a known anorexic, presented with complaints of soreness and weakness in bilateral

    lower extremities, as well as difficulty ambulating for 2-3 days. She was hypotensive and bradycardic at

    an outside hospital and was given two normal saline boluses prior to transfer to our pediatric ICU for

    further management. Her initial BMI was 11.7 and was at 60% of ideal body weight. Shortly after

    initiating feeds she was found to have hypokalemia, hypophosphatemia and hypomagnesemia consistent

    with refeeding syndrome. On hospital day three, our patient was found to have a new-onset murmur,which was assessed with an echocardiogram and found to be caused by moderate-to-severe mitral

    regurgitation.

    ResultsWhen electrolyte abnormalities were first appreciated oral supplementation was commenced with

    potassium phosphate. This was increased due to continued deficiencies, and magnesium was also

    supplemented. All electrolyte abnormalities were corrected by hospital day three. Feeding regimen was

    continued with modest increases in caloric intake during the first week of refeeding. The patient never

    developed significant symptoms from mitral regurgitation and did not sustain right heart failure because

    of early diagnosis, appropriate management, and careful observation.

    ConclusionThis case of an adolescent female with severe malnutrition secondary to anorexia had severalrisk factors for the development of refeeding syndrome and its complications. However, those risk factors

    were accounted for during treatment and our patient still developed refeeding syndrome, likely due to the

    two boluses received prior to admission to the ICU. These fluids were not needed due to the

    compensation that is observed in patients with anorexia nervosa. Fortunately, there were no further

    complications and the mitral regurgitation remained asymptomatic, but had the potential to cause more

    significant effects, such as heart failure.

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    ABSTRACT 22

    Nasopharyngeal Teratoma and Imperforate Anus in a Neonate: Case Report

    Parampreet Kathuria DO1, WasylSzeremeta MD2, Shanthy Sridhar MD3

    1Department of Pediatrics, Stony Brook University School of Mediciner, 2Division of Surgery and

    Department of Pediatrics, Stony Brook University School of Medicine,3Division of Neonatology,

    Department of Pediatrics, Stony Brook University School of Medicine

    Teratomas arising from the nasopharynx are rare occuring approximately in 1 out of every 4,000 live

    births. These tumors are composed of the three germinal layers - ectoderm, mesoderm, and endoderm -

    and are characterized by poor cell differentiation. Teratomas of the head and neck account for 2-9% of all

    cases of teratomas in children. Nasopharyngeal teratomas usually cause immediate dyspnea at birth

    requiring urgent intubation and ventilatory support. Resolution of symptoms requires surgical excision

    after which a low recurrence rate of nasopharyngeal tumors has been reported.

    Background

    We report a female neonate born at 37 weeks gestation who was found to have a large nasopharyngeal

    mass undiagnosed in utero, an imperforate anus, and a laryngeal web. She was born via C-section due to

    decreased fetal growth and significant polyhydramnios. She was born floppy and no respirations were

    noted. There was a significant airway obstruction and she was successfully intubated after which physical

    examination revealed a hard mass on the palate and a clear cystic mass on the soft palate. A CT scan of

    her brain and facial bones confirmed a mass extending into the nasopharyngeal region. She was taken to

    the OR for resection of mass and tracheostomy. Intraoperative findings with direct laryngoscopy revealed

    a 2.5 to 3 cm hard mass emanating off the inferior edge of the left palate. She wasextubatedintraopertively and her tracheostomy was reversed at one week of life after which she developed

    respiratory distress. She was brought back to the OR when a bronchoscopy revealed a laryngeal web and

    subglottic stenosis. At this time, the laryngeal web was resected and she was retrached. On initial exam

    she was thought to have an anteriorly displaced anus. At 6 weeks of age she underwent an anoplasty for

    an imperforate anus with perineal fistula found on intraoperative examination as well as PEG tube

    placement. She received physical, occupational, and speech therapy while in the hospital and these

    services were continued after being discharged. At 7 months she appears to be doing well with no

    evidence of tumor recurrence.

    Case

    Teratomas of the head and neck are rare congenital neoplasms that arise from pluripotent cells that

    account for less than 2% of reported cases of congenital teratomas. The recommended management of

    nasophargnealteratomas is surgical excision. Congenital anomalies have been reported in patients with

    nasopharyngeal teratomas such as a cleft palate, pneumothoraces, unilateral choanal atresia, and inguinal

    hernias. To the best of our knowledge, this is the first report of a child with a nasopharyngeal teratoma,

    imperforate anus requiring anoplasty, and a laryngeal web requiring resection.

    Conclusion

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    ABSTRACT 23

    Pain-Reducing Interventions During Pediatric Venipuncture and IV Insertion Leads to Increased

    Parent Satisfaction

    Sushma Krishna MD, Carolyn Milana MD, Catherine Messina PhD, Grace Propper RN, MS, Michael

    Attard MA, CCLS

    Topical anesthesia during painful procedures leads to lower pain scores in children; however the effect on

    parent satisfaction has not been characterized. The purpose of this study is to determine the effect of

    various pain-reducing interventions during painful pediatric procedures on parent satisfaction.

    Background

    Parent(s) of children undergoing either IV insertion or venipuncture on the general pediatric floor at

    Stony Brook Long Island Childrens Hospital were included. Children received no intervention, topical

    anesthetic alone, distraction techniques alone, or topical anesthetic with distraction techniques.

    Distraction techniques included child life specialist, nursing distraction, and child life box. Patient age,

    gender, and type(s) of intervention used were recorded. Parent(s) reported whether the intervention(s)

    increased their satisfaction with their hospital stay based on a 4 point scale (1Strongly Disagree, 2

    Disagree, 3Agree, 4Strongly Agree).

    Methods

    Parents of 79 children, ages 0 to 20 years, undergoing IV insertion or venipuncture were included; 49.4%

    females (n=39), 45.6% males (n=36), and 5.1% (n=4) gender was not reported. Sixty patients (75.9%)

    received pain-reducing intervention(s) and 19 (24.1%) patients received no intervention.

    Results

    Parents of children who received pain-reducing interventions were more likely to agree or strongly agree

    that this intervention increased satisfaction with their childs hospital stay as compared to parents of

    children who received no intervention (85.0% versus 52.7%, p

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    ABSTRACT 24

    SYSTEMATIC SURVEY OF INTUBATION SUCCESS AND OPPORTUNITY IN A DUTY-

    HOUR LIMITED PEDIATRIC AND NEONATAL TRAINING PROGRAM

    1Patricia Mele, DNP, 2Loren Murphy, MD, 2Shetal Shah, MD, and 2Aruna Parekh MD1Department of Pediatrics and 2

    Division of Neonatal Medicine, Stony Brook University School of

    Medicine

    Neonatal intubation is critical in resuscitation. Duty-hour restrictions limit real-life training opportunities,

    increasing reliance on mid-level providers (NNP). The 2012 Neonatal Resuscitation Program (NRP)

    guidelines emphasize simulation training to improve resuscitation proficiency. Our trainees receive NRP

    certification with reinforcement during neonatal intensive care unit (NICU) rotation. Assessment of

    airway skills in this post-training era has not been evaluated.

    Background

    Objective: To survey intubation practice patterns in a tertiary care Level III NICU.

    From Mar to Oct 2011, a sample of intubations performed by NICU providers was independently

    evaluated. Data on trainee level, intubation success, reason for failure, location of intubation &

    proficiency (0-5 Likert Scale) were collected. Demographic data included post-menstrual age & weight at

    intubation. Data were analyzed using Fisher's Exact Test and ANOVA with Bonferroni correction for

    multiple comparison.

    Methods

    282 intubations were analyzed with an overall first-attempt success rate of 52.1%. This rate was higher in

    the Delivery Room (DR), (62% vs. 47%, p

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    ABSTRACT 25

    Characteristics and outcomes of pediatric rapid response teams before and after automation by a

    pediatric early warning score (PEWS) system

    Polikoff, Lee; Panesar, Rahul; Mills, Barbara; Harris, David; Greene, William

    The Pediatric Early Warning Score (PEWS) System was created to help identify unstable patients prior to

    a life-threatening event. Rapid Response Teams (RRTs) were developed to assist in the evaluation and

    management of these patients. Since December 2008, we have automated the activation of RRTs by a

    PEWS of 5 or greater (red score).

    Introduction

    We aim to identify differences in characteristics of RRT calls before and after automation by the PEWS

    System.

    Hypothesis

    We compared RRT data compiled from 2008 to 2010, with 44 RRTs recorded before automation and 69

    RRTs after automation by the PEWS System. Chi-Square analysis was performed; P-value

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    ABSTRACT 26

    Successful Remission of Dense Deposit Disease (DDD) with Plasmapheresis: A Case Report

    Sheena Sharma, MD1, ChristophLicht, MD2, Katarina Supe-Markovina, MD

    1

    1Department of Pediatrics, Stony Brook University School of Medicine; 2

    Pediatric Nephrology, Hospitalfor Sick Children

    DDD is an extremely rare condition affecting 2-3 persons per million in the general population. The mostcommon etiology is due to C3 nephritic factor (C3NF), an IgG autoantibody which stabilizes C3convertase (C3bBb), resulting in uncontrolled activation of the alternative pathway of complement.Remnants of C3 are deposited in the glomerular basement membrane causing glomerular injury.Approximately 50% of patients develop end-stage renal disease within 10 years. Due to its rarity, there

    are no evidence based guidelines for the treatment of DDD. We present the case of a 15 year oldCaucasian male with DDD secondary to C3NF who achieved remission with short-term plasmapheresistherapy.

    Background

    Our patient presented with gross hematuria and proteinuria. A native kidney biopsy revealed DDD.Components of the complement cascade were measured. C3 was found to be low and C3NF waselevated. In order to remove the pathologic antibody, pheresis treatments were initiated. Treatmentswere performed with the Cobe Spectra Collect Flow Path using citrate dextrose containing heparin for

    anticoagulation and 5% albumin as replacement fluid. Our patient had a total of 18 treatments over 6months; weekly for 3 months then bimonthly for an additional 3 months. C3 levels and proteinuria weremonitored bimonthly, and C3NF was monitored prior to the initiation of treatments, mid-way throughtreatments, and following cessation of treatments.

    Methods

    C3 and proteinuria normalized after 6 months of plasmapheresis. C3NF initially decreased whilereceiving treatments, but increased once stopped. Despite this, he remains in clinical remission defined asurine protein/creatinine

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    ABSTRACT 27

    The Stony Brook Parent Handbook: From Birth to 5 Years

    Michael R Stern MD, Traci Downs MD

    There is currently a staggering amount of medical information of varying quality that is readily available

    both online and in print. There is a need for a simplified starting point for the typical first-time parentseeking accurate healthcare information.

    Background

    To create a concise, high-yield and easily understandable handbook for parents. The handbook will aimto serve as a starting point for new parents that will complement but not replace the advice of

    pediatricians or that found in more comprehensive parenting guides.The handbook will review vaccines,standard anticipatory guidance, treatment of common problems and basic first aid. It will also provide alisting of suggested sources for more detailed information. When complete, the handbook will be printed

    in an economical format and provided free of charge to any interested parent of a patient of the StonyBrook Long Island Childrens Hospital.

    Objectives

    Content and format of anticipatory guidance was synthesized from reviewing a breadth of the available

    literature. Major print sources include several of the American Academy of Pediatrics (AAP) topicspecific handouts and comprehensive parenting books. Other sources include the What to Expect series

    by Murkoff and Mazel and Caring for Kids: The Complete Guide to Childrens Health from theHospital For Sick Children in Toronto, Canada. Electronic sources included parent targeted websites ofthe AAP (

    Methods

    www.healthychildren.org), the DuPont Childrens Hospital (www.kidshealth.org)and the

    Hospital For Sick Children (www.aboutkidshealth.ca). Any discordance was settled through personalcommunication between the author and various board-certified pediatricians. Primary assessment ofreadability was performed using the SMOG score for readability. This estimates the reading level

    required to achieve 100% comprehension of the material in question. Secondary assessment of readabilityand content was assessed by directly surveying physicians and laypersons. Separate surveys were

    prepared for attending and resident physicians and parents. Drafts of the handbook were distributed withsurveys to 15 attendings, 15 residents and 30 parents.

    Primary assessment with SMOG score revealed that the first draft required greater than a 12Results

    thgrade

    reading level to comprehend most of the content. After a comprehensive revision and rewrite; the seconddraft scores between the 6thand 7th

    grade reading level. At the time of abstract submission, secondary

    assessment via physician and parent survey has yet to yield sufficient results for statistical evaluation.Preliminary survey response from 6 Attending Physicians and 5 Resident Physicians revealed that 100%would give a revised and finalized version of this handbook to their patients. It is anticipated that

    continued response via returned surveys will provide valuable insights to be included in a final revisionand reformatting of the book.

    http://www.healthychildren.org/http://www.healthychildren.org/http://www.healthychildren.org/http://www.kidshealth.org/http://www.kidshealth.org/http://www.kidshealth.org/http://www.aboutkidshealth.ca/http://www.aboutkidshealth.ca/http://www.aboutkidshealth.ca/http://www.aboutkidshealth.ca/http://www.kidshealth.org/http://www.healthychildren.org/